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About HCHWA-D

Katwijk Disease: a rare hereditary cerebral disorder

HCHWA-D, also known as the 'Katwijk disease', is a hereditary form of cerebral haemorrhaging.

HCHWA-D?

HCHWA-D is the acronym for Hereditary Cerebral Haemorrhage With Amyloidosis. The 'D' stands for 'Dutch type'. In Dutch it is called: erfelijke hersenbloedingen met amyloïdose (accumulation of an unusual protein) - Dutch type. HCHWA-D finds its origins in Katwijk (a town located on the Dutch North Sea) and is mainly found in a small number of Dutch families and some emigrants in Australia with Dutch ancestral roots.

Cerebral Haemorrhaging

A distinct trait of the disease HCHWA -D is the accumulation of proteins (amyloids) in the arteries of the brain. This causes cerebral haemorrhaging. The first stroke usually occurs between the ages of 45 and 65, however, incidents have been reported of younger as well as older patients. The severity as well as resulting effects of the haemorrhages can vary greatly. Also, the number of attacks differs per person. HCHWA-D can be related to dementia.

Read more on:

  • The characteristics and development of the disease: What is HCHWA-D?
  • The differences and similarities between HCHWA-D and CAA (Cerebral Amyloid Angiopathy)
  • Whether or not someone is a carrier of the disease: Do I have HCHWA-D?
  • What the rehabilitation possibilities are after a stroke:  What do I do now? 

Heredity

The gene responsible for HCHWA-D was discovered in 1990. The inheritance of the gene is autosomal dominant (non-sex-related). This means that there is a 50% chance that a child will inherit the disorder from a parent who carries the HCHWA-D gene. At the moment, there are still no possibilities of preventing or curing this disease.

More information on :

  • The inheritance of HCHWA-D can be found in "Is HCHWA-D hereditary?"
  • Having children without HCHWA - D can be found under "Having Children"

*Katwijk Disease

HCHWA-D is commonly referred to as 'Katwijk disease'. Two out of three HCHWA-D affected families live or used to live in, or around, Katwijk. The third family currently resides in Scheveningen. It is possible, though not confirmed, that all three families may have descended from a common ancestor and are therefore distant relatives.